Felty's Syndrome, Insights and Updates

Abstract

Felty's syndrome (FS) is characterized by the triad of seropositive rheumatoid arthritis (RA) with destructive joint involvement, splenomegaly and neutropenia. Current data shows that 1-3 % of RA patients are complicated with FS with an estimated prevalence of 10 per 100,000 populations. The complete triad is not an absolute requirement, but persistent neutropenia with an absolute neutrophil count (ANC) generally less than 1500/mm3 is necessary for establishing the diagnosis. Felty's syndrome may be asymptomatic but serious local or systemic infections may be the first clue to the diagnosis. FS is easily overlooked by parallel diagnoses of Sjӧgren syndrome or systemic lupus erythematosus or lymphohematopoietic malignancies. The role of genetic (HLA DR4) is more prominent in FS in comparison to classic rheumatoid arthritis. There is large body of evidence that in FS patients, both cellular and humoral immune systems participate in neutrophil activation, and apoptosis and its adherence to endothelial cells in the spleen. It has been demonstrated that proinflammatory cytokines may have inhibitory effects on bone marrow granulopoiesis. Binding of IgGs to neutrophil extracellular chromatin traps (NET) leading to neutrophil death plays a crucial role in its pathophysiology. In turn, "Netting" neutrophils may activate auto-reactive B cells leading to further antibody and immune complex formation. In this review we discuss on basic pathophysiology, epidemiology, genetics, clinical, laboratory and treatment updates of Felty's syndrome.

Keywords: Cytopenia; Felty’s syndrome; LGL syndrome; neutropenia; rheumatoid arthritis; splenomegaly; treatment..

Fig. (1)

Hypocellular marrow with myeloid hypoplasia in FS.

Fig. (2)

LGL cells in peripheral blood (from one of our mutual patients)

Fig. (3)

LGL cells in bone marrow.